Hyper-immunoglobulin E syndrome (Job syndrome) is a rare primary immunodeficiency with variable presentation, characterized by recurrent infections, facial dimorphism, eczema, scoliosis, joint hyper-extensibility, pathologic fractures, very high IgE (>2000 IU/mL), severe eosinophilia and variable impaired T cell function. We present a case of Hyperimmunoglobulin E syndrome in neonate with review of the literature. J Microbiol Infect Dis 2013; 3(3): 144-146.